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Steven A Newman, Justin Nathaniel Karlin, Jose Gurrola; Allergic Fungal Sinusitis-Masquerade Syndrome and Delay in Diagnosis. Invest. Ophthalmol. Vis. Sci. 2017;58(8):5149.
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© ARVO (1962-2015); The Authors (2016-present)
Similar to the destructive chronic allergic broncho-pulmonary aspergillosis, allergic fungal sinusitis (AFS) is an IgE-mediated inflammatory disease of the paranasal sinuses which may produce an expansive erosive syndrome causing damage to surrounding tissues. Patients with AFS often present with orbital and neuro-ophthalmic signs including proptosis, dystopia, and decreased central acuity due to compressive optic neuropathy. These findings may mistakenly be attributed to other causes of pathology, including thyroid orbitopathy, and may lead to a substantial delay in diagnosis and in what may be an important surgical intervention.
We report an individual case presenting as “thyroid orbitopathy” with proptosis, lid retraction, restricted motility, and optic neuropathy initially responsive to IV pulse steroids for presumed thyroid orbitopathy. The patient was later found to have evidence of allergic fungal sinusitis leading to endoscopic decompression. Review of our previous experience revealed 9 cases coded as allergic fungal sinusitis.
These patients demonstrate that allergic fungal sinusitis is an uncommon but not rare form of chronic rhinosinusitis that often presents with expansion of the involved sinuses often affecting the orbit and visual pathways. In our series bone erosion is common, as is an inflammatory reaction and eosinophilia. These patients may initially respond to steroid therapy, further delaying diagnosis. Surgical decompression and appropriate anti-inflammatory treatment often results in marked improvement in orbital, ocular, and cranial nerve function, even without anti-fungal treatment for invasive disease.
Allergic fungal sinusitis needs to be part of the differential diagnosis of orbital pathology, even without a previous history of sinus disease. Cases may masquerade as more common orbital pathology (including thyroid orbitopathy) although most cases of allergic fungal sinusitis do have other evidence to suggest sinus disease. With that said, patients may also present with what appear to be strictly orbital pathology. Imaging is critical to the diagnosis and perhaps should be considered earlier. The advent of endoscopic sinus surgery has substantially improved our ability to deal with these cases in a minimally invasive form.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.
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